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Rev. colomb. cir ; 38(2): 363-368, 20230303. fig
Article in Spanish | LILACS | ID: biblio-1425217


Introducción. El angiosarcoma es una neoplasia vascular originada a nivel del endotelio, de baja frecuencia, conocido por su agresividad y crecimiento acelerado. Alcanza solo el 1 al 2 % del total de los sarcomas. La presentación gastrointestinal es infrecuente y su incidencia es poco conocida debido al escaso reporte de casos en la literatura. Suele identificarse en etapas avanzadas debido a la dificultad del diagnóstico histopatológico por sus características morfológicas, siendo necesario aplicar tinciones especiales o estudio inmunohistoquímico. Caso clínico. Por su interés y singularidad, presentamos el caso de un paciente masculino de 54 años, quien consultó con hemorragia digestiva profusa, anemización y requerimiento de terapia transfusional. Resultados. Durante el proceso diagnóstico, en la endoscopia encontraron diversas lesiones multifocales que se extendían por gran parte del tracto gastrointestinal. El estudio histopatológico mostró angiosarcoma gastrointestinal. Discusión. El angiosarcoma del tracto gastrointestinal es extremadamente infrecuente, de difícil diagnóstico y bajas posibilidades de manejo curativo, con opciones terapéuticas limitadas, lo que configura un mal pronóstico a corto plazo

Introduction. Angiosarcoma is a vascular neoplasm originating from endothelial cells, known for its aggressiveness, accelerated growth and reduced frequency. Reach only 1 to 2% of total sarcomas. Gastrointestinal presentation is extremely rare, the true incidence is poorly known, due to the limited reports of this entity in the literature. It is usually identified in advanced stages in view of the difficulty of the histopathological diagnosis, attributable to its morphological characteristics, being necessary to apply special stains or immunohistochemical study. Case report. Due to their interest and uniqueness, we present the case of a 54-year-old male patient, who presented with a profuse gastrointestinal bleeding, anemia, and requirement for transfusion therapy. Results. The endoscopy detected several multifocal lesions that extended most of the gastrointestinal tract. The histopathological study showed gastrointestinal angiosarcoma. Discussion. Angiosarcoma of the gastrointestinal tract is extremely uncommon, difficult to diagnose and has low possibilities of curative management, with limited therapeutic options, which configures a poor prognosis in the short term

Humans , Gastrointestinal Neoplasms , Hemangiosarcoma , Sarcoma , Lymphatic Vessel Tumors , Gastrointestinal Hemorrhage , Neoplasms, Vascular Tissue
Chinese Journal of Pathology ; (12): 1132-1137, 2023.
Article in Chinese | WPRIM | ID: wpr-1012377


Objective: To investigate the clinical manifestations, histomorphology, and differential diagnosis of primary hepatic angiosarcoma. Methods: Nine cases of primary hepatic angiosarcoma diagnosed in the Department of Pathology, the First Affiliated Hospital of Nanjing Medical University from January 2014 to December 2021 were collected, including biopsy and surgical specimens. The histomorphology, clinical, and radiologic findings were analyzed. The relevant literature was also reviewed. Results: There were six males and three females, aged 30 to 73 years (mean 57 years). Grossly, the growth pattern of the tumor was classified as either mass formation or non-mass formation (sinusoidal). Microscopically, the mass-forming primary hepatic angiosarcoma were further subdivided into vasoformative or non-vasoformative growth patterns; and those non-vasoformative tumors had either epithelioid, spindled, or undifferentiated sarcomatoid features. Sinusoidal primary hepatic angiosarcoma on the other hand presented with markedly dilated and congested blood vessels of varying sizes, with mild to moderately atypical endothelial cells. Follow-up in all nine cases revealed 8 mortality ranging from 1 to 18 months (mean 5 months) from initial diagnosis. One patient was alive with disease within a period of 48 months. Conclusions: Primary hepatic angiosarcoma is a rare entity with a wide spectrum of histomorphology, and often misdiagnosed. It should be considered when there are dilated and congested sinusoids, with overt nuclear atypia. The overall biological behavior is aggressive, and the prognosis is worse.

Male , Female , Humans , Hemangiosarcoma/diagnosis , Endothelial Cells/pathology , Liver Neoplasms/surgery , Prognosis , Biopsy
Chinese Journal of Oncology ; (12): 74-81, 2023.
Article in Chinese | WPRIM | ID: wpr-969808


Objective: To evaluate the efficacy and safety of different medical treatment in advanced or unresectable angiosarcoma. Methods: This study was a single-center retrospective clinical study. Fifty-five advanced or unresectable angiosarcoma patients treated in Sun-Yat Sen University Cancer Center from January 2005 to August 2020 were enrolled. There were 34 patients who received first-line doxorubicin-based chemotherapy (doxorubicin group), 12 patients received first-line doxorubicin or liposome doxorubicin plus paclitaxel or albumin bound paclitaxel chemotherapy (combination therapy group), and 4 patients received first-line paclitaxel-based treatment (paclitaxel group). There were 6 patients who received anti-angiogenesis targeted therapy, another 2 patients received anti-PD-1 antibody plus anti-angiogenesis targeted therapy. Targeted therapy and immunotherapy plus targeted therapy included 5 cases of first-line therapy and 3 cases of second-line therapy. The therapeutic effect was evaluated by RECIST 1.1 standard. The adverse reactions were evaluated by CTCAE4.0 standard. Kaplan-Meier survival analysis was evaluated with Log rank test. Cox proportional hazard model was used to analyze the influencing factors. Results: There were 18 patients achieved partial response (PR) in 34 patients in the doxorubicin group, median progression-free survival (mPFS) was 4.5 months, and median overall survival (mOS) was 15 months. Four patients achieved PR in 12 patients in the combination therapy group, mPFS and mOS were 4 months and 19 months. Two patients achieved PR in 4 patients in the paclitaxel group, mPFS and mOS were 3 months and 9 months. However, only 1 in 6 patients achieved PR for anti-angiogenesis targeted therapy, mPFS and mOS were 3 months and 16 months. Two patients who received anti-PD-1 immunotherapy combined with anti-angiogenesis targeted therapy acquired PR for 17 months and more than 16 months. Median PFS (7.5 months) were longer in those with primary liver, lung and spleen angiosarcoma than in those with other primary site (3.0 months, P=0.028). The mOS (20 months) was longer in females than that in males (12 months, P=0.045). Primary tumor site, sex, age and treatment were not independent prognostic factors for angiosarcoma patients (P>0.05). Grade 3-4 cardiac toxicity was found in 2 patients in the combination therapy group. Conclusions: Doxorubicin-based and paclitaxel-based chemotherapy are the most important treatment for advanced angiosarcoma. Potential efficacy for targeted therapy combined with anti-PD-1 immunotherapy are showed in some patients with long duration of response and moderate adverse event.

Male , Female , Humans , Hemangiosarcoma , Retrospective Studies , Paclitaxel/adverse effects , Doxorubicin/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/adverse effects
Chinese Journal of Hepatology ; (12): 729-735, 2023.
Article in Chinese | WPRIM | ID: wpr-986202


Objective: To investigate the clinicopathological features, treatment, and prognosis of hepatic angiosarcoma. Methods: Clinicopathological data and prognostic conditions of 18 cases with hepatic angiosarcoma were collected retrospectively. The recurrence-free survival rate and overall survival rate were calculated by the Kaplan-Meier method. A Cox regression analysis was used to explore the survival-related risk factors. Results: There were 12 male and 6 female patients, with an average age of 57 (37 ~ 70) years. The tumor's average diameter was 8.40 (2.00 ~ 18.00) cm. Seven cases had multiple tumors, while two cases had large vessel tumor thrombuses. Microscopically, the tumor tissues were irregularly anastomosed, with vascular lacunar or solid bundle-like weaving, and the tissue morphology mimicked capillary hemangioma, cavernous hemangioma, or angioepithelioma, while tumor cells were spindle-shaped or epithelioid, lined with hobnails in the lumen, or formed papillary structures in the lumen. The proportion of highly, moderately, and poorly differentiated tumors was 4:8:6, with six cases having clear tumor boundaries, eight having microvascular tumor thrombi, and sixteen having blood lake formation. Different levels of expression of CD31, CD34, erythroblast transformation-specific related genes, and Fli-1 markers were demonstrated in all of the cases. Four cases had a P53 mutation, and six cases had Ki-67 > 10%. During the follow-up period of 0.23-114.20 months, the five-year recurrence-free survival rate and overall survival rate were 16.7% and 37.2%, respectively. Cox regression multivariate analysis showed that preoperative symptoms and multiple tumors were significant risk factors for recurrence-free survival, while preoperative symptoms and Ki-67 > 10% were significant risk factors for overall survival. Conclusion: Hepatic angiosarcoma is a rare hepatic mesenchymal tumor with high malignancy and a poor prognosis. Pathological morphology and immunohistochemical marker combinations are needed for a definite diagnosis. However, the complexity of angiosarcomas' histological and cytological conformations and the overlap of pathological features with benign vascular tumors, sarcomas, and carcinomas pose difficulties in the differential diagnosis. Thus, the only effective ways to prolong survival are early detection and radical surgical resection.

Humans , Male , Female , Middle Aged , Hemangiosarcoma , Ki-67 Antigen , Retrospective Studies , Biomarkers, Tumor/metabolism , Prognosis , Liver Neoplasms/pathology
Chinese Journal of Pathology ; (12): 599-605, 2023.
Article in Chinese | WPRIM | ID: wpr-985739


Objective: To investigate the clinicopathological and molecular features of primary cardiac angiosarcoma (PCAS), and to analyze the correlation between KDR mutation and the clinicopathological features of PCAS. Methods: Thirteen cases of PCAS were collected at Beijing Anzhen Hospital, Capital Medical University from January 2007 to December 2021. The clinicopathological features, diagnosis, differential diagnosis and outcome were retrospectively analyzed. KDR mutation was detected by next-generation sequencing (NGS) and then the expression of KDR (VEGFR2) was determined by immunohistochemistry (IHC), with review of relevant literatures. Results: There were eight males and five females with a mean age of 45 years. The primary tumor was in the right atrium in 10 cases, left atrium in two cases and right ventricle in one case. The histomorphology was mainly poorly differentiated angiosarcoma (11 cases), with highly pleomorphic spindle or round cells in solid sheets, brisk mitotic activity and extensive necrosis. Vascular lumen formation was observed in two cases of high to moderate differentiation, and biphenotypic differentiation was seen in five cases. IHC staining showed CD34, CD31, Fli1, ERG and vimentin were diffusely positive, pan-cytokeratin was positive, Ki-67 index ranged from 3% to 90%, which was positively correlated with the differentiation degree and grade of the PCASs (P<0.05). At the end of follow-up period, one patient was alive, two patients were lost to follow-up, and the remaining 10 patients had an average survival time of 4.6 months. Finally, NGS sequencing was performed on seven samples after screening, and the results showed that KDR and NF1 mutations were both present in three cases. VEGFR2 expression had no significant correlation with the differentiation degree and grade of PCAS (P>0.05), and it was not related to KDR mutation. Conclusions: PCASs mainly occur in the right atrium, and are mainly poorly differentiated. Ki-67 index is helpful to assess the degree and grade of tumor differentiation. The occurrence and development of PCAS may be related to the pathway involved in KDR mutation, but KDR mutation has no clear correlation with clinicopathological characteristics of PCAS, and immunohistochemical staining can not replace gene detection to determine whether the tumor had KDR mutation.

Male , Female , Humans , Middle Aged , Hemangiosarcoma/genetics , Retrospective Studies , Ki-67 Antigen , Immunohistochemistry , Molecular Biology , Biomarkers, Tumor/analysis
Rev. otorrinolaringol. cir. cabeza cuello ; 82(4): 459-465, dic. 2022. ilus
Article in Spanish | LILACS | ID: biblio-1431936


Los tumores malignos de cavidad nasal y cavidades paranasales son poco frecuentes y sus síntomas de presentación suelen ser unilaterales e inespecíficos; éstos incluyen: epistaxis, rinorrea y obstrucción nasal. Encontramos una amplia variedad histológica de tumores, entre ellos tenemos el angiosarcoma nasal, que es considerado una rareza y el diagnóstico definitivo lo da la confirmación anatomopatológica complementado con estudios inmunohistoquímicos. Su modalidad de tratamiento incluye múltiples opciones, pero la cirugía radical temprana con márgenes negativos asociado a radioterapia adyuvante, ofrece el mejor pronóstico. Se presenta a un paciente adulto masculino con un angiosarcoma nasal izquierdo que fue detectado de forma precoz y se manejó con cirugía radical y radioterapia.

Malignant tumors of the nasal cavity and paranasal cavities are rare, while presenting symptoms are usually unilateral and nonspecific like, epistaxis, rhinorrhea, and nasal obstruction. There are many histological varieties of tumors, among them we have nasal angiosarcoma, which is considered a rarity pathology. The definitive diagnosis is given by biopsy confirmation complemented with immunohistochemical studies. Its treatment modality includes multiple options, but early radical surgery with negative margins associated with adjuvant radiotherapy offers the best prognosis. We present a male adult patient with a left nasal angiosarcoma that was detected early and managed with radical surgery and radiotherapy.

Humans , Male , Adult , Nose Neoplasms/pathology , Nose Neoplasms/diagnostic imaging , Hemangiosarcoma/pathology , Hemangiosarcoma/diagnostic imaging , Nasal Cavity/pathology , Tomography, X-Ray Computed/methods , Nose Neoplasms/surgery , Nasal Surgical Procedures/methods , Hemangiosarcoma/surgery
Rev. colomb. cir ; 38(1): 176-181, 20221230. fig
Article in Spanish | LILACS | ID: biblio-1417733


Introducción. El angiosarcoma primario de la mama es una neoplasia maligna derivada de las células endoteliales de los vasos sanguíneos, potencialmente agresiva independientemente de su grado histológico, por lo que su pronóstico es malo. Su diagnóstico prequirúrgico es difícil, ya que las características clínicas e imagenológicas son inespecíficas, y el diagnóstico definitivo únicamente se realiza por estudios de patología. Para su tratamiento generalmente se requiere de resección quirúrgica, radioterapia y, ocasionalmente, quimioterapia.Caso clínico. Paciente de 49 años sin antecedentes, que consultó por cuadro clínico de 5 meses de evolución de aparición y rápido crecimiento de masa en mama izquierda. Se realizaron estudios imagenológicos que reportaron lesión BIRADS 4a y diagnóstico histológico de lesión vascular con atipía, por lo cual fue llevada a mastectomía simple, con informe final de patología de angiosarcoma primario de mama; tuvo que ser reintervenida por márgenes positivos. Completó 33 ciclos de radioterapia y dos años después de la cirugía presentó cambios inflamatorios en la cicatriz quirúrgica, de la cual se tomó biopsia con reporte de lesión vascular atípica, por lo que fue operada nuevamente, con reporte histológico negativo para angiosarcoma residual. Actualmente la paciente está en seguimiento imagenológico, sin evidencia de recaída tumoral. Conclusión. Los angiosarcomas primarios de la mama son neoplasias raras y muy agresivas, independientemente de su grado histológico, por lo cual es importante hacer un diagnóstico histológico y tratamiento oncológico oportunos.

Introduction. Primary breast angiosarcoma is a malignant pathology derived from the endothelial cells of the blood vessels of the breast. They are potentially aggressive regardless of histological grade, reason why its prognosis is poor and treatment requires surgical resection plus radiation therapy and occasionally chemotherapy depending on the degree. Its pre-surgical diagnosis is difficult since the clinical and imaging characteristics are nonspecific, and the definitive diagnosis is only made by means of pathology studies. Clinical case. A 49-year-old patient was admitted to the breast surgery outpatient clinic due to clinical symptoms of 5 months of evolution consisting of the appearance of a painful mass in the left breast. Imaging of the lesion with ultrasound report BIRADS 4a and a tricot biopsy was taken with histological diagnosis of vascular lesion with atypia. It was decided to take the patient to a simple mastectomy, with a final report of breast angiosarcoma but with a margin compromised by a tumor for which she was reoperated. She received 33 cycles of radiotherapy and continued in follow-up for two years. During this period, the patient presented inflammatory changes in the surgical scar for which a punch biopsy was done with histological report of vascular atypical lesion. Surgical resection was performed with final report of negative pathology for residual angiosarcoma. Nowadays the patient continues imaging follow-up without evidence of a tumour relapse. Conclusion. Primary breast angiosarcomas are a rare malignant pathology, very aggressive regardless of its histological grade, for which it is important to perform a timely histological diagnosis and oncological treatment

Humans , Hemangiosarcoma , Radiotherapy , Breast Neoplasms , Immunohistochemistry , Mastectomy
Arq. Ciênc. Vet. Zool. UNIPAR (Online) ; 25(2): e8895, jul-dez. 2022. ilus
Article in Portuguese | LILACS, VETINDEX | ID: biblio-1399615


O hemangiossarcoma é uma neoplasia mesenquimal maligna agressiva com elevada taxa de morbidade e de mortalidade em cães e gatos; que se desenvolve mais frequentemente em baço, fígado, coração, ossos além de poder manifestar metástases regionais. O presente relato tem por objetivo descrever um caso de um canino diagnosticado com hemangiossarcoma hepático em lobo caudado submetido a uma lobectomia total. A técnica cirúrgica consistiu na utilização da sutura de guilhotina modificada na base do lobo acometido utilizando fio de polidioxanona e, com o auxílio de um bisturi elétrico unipolar, e respeitando o distanciamento de 0,5 cm da sutura, foi realizada a lobectomia. Após a remoção do lobo, foi fixada uma esponja hemostática de colágeno na região da incisão como forma de auxílio no controle hemorrágico. Foi indicado também a realização de tratamento quimioterápico adjuvante, entretanto o tutor do animal optou pela não realização da mesma. Ainda assim, o paciente do presente relato obteve uma boa resposta ao procedimento, e o tutor ficou muito satisfeito, relatando que houve uma grande melhora na qualidade de vida do animal e que ele não sentia mais dor, voltando a ter o seu comportamento habitual.(AU)

Hemangiosarcoma is an aggressive malignant mesenchymal neoplasm with a high rate of morbidity and mortality in dogs and cats; which develops more frequently in the spleen, liver, heart, bones, in addition to being able to manifest regional metastases. The present report aims to describe a case of a canine diagnosed with hepatic hemangiosarcoma in the caudate lobe submitted to a total lobectomy. The surgical technique consisted of using a modified guillotine suture at the base of the affected lobe using polydioxanone thread and, with the aid of a unipolar electric scalpel, and respecting the distance of 0.5 cm from the suture, lobectomy was performed. After removing the lobe, a hemostatic collagen sponge was fixed in the region of the incision as an aid in hemorrhagic control. Adjuvant chemotherapy treatment was also indicated, however the animal's tutor chose not to perform it. Even so, the patient in the present report had a good response to the procedure, and the tutor was very satisfied, reporting that there was a great improvement in the animal's quality of life and that he no longer felt pain, returning to his usual behavior.(AU)

El hemangiosarcoma es una neoplasia mesenquimatosa maligna agresiva con una alta tasa de morbilidad y mortalidad en perros y gatos; la cual se desarrolla con mayor frecuencia en bazo, hígado, corazón, huesos, además de poder manifestar metástasis regionales. El presente reporte tiene como objetivo describir un caso de un canino con diagnóstico de hemangiosarcoma hepático en el lóbulo caudado sometido a una lobectomía total. La técnica quirúrgica consistió en utilizar una sutura de guillotina modificada en la base del lóbulo afectado con hilo de polidioxanona y, con la ayuda de un bisturí eléctrico unipolar, y respetando la distancia de 0,5 cm de la sutura, se realizó la lobectomía. Después de retirar el lóbulo, se fijó una esponja hemostática de colágeno en la región de la incisión como ayuda para el control hemorrágico. También se indicó tratamiento de quimioterapia adyuvante, sin embargo el tutor del animal optó por no realizarlo. Aun así, el paciente del presente reporte tuvo una buena respuesta al procedimiento, y el tutor quedó muy satisfecho, informando que hubo una gran mejoría en la calidad de vida del animal y que ya no sintió dolor, volviendo a su comportamiento habitual.(AU)

Animals , Female , Dogs , Anterior Temporal Lobectomy/methods , Hemangiosarcoma/surgery , Liver Neoplasms/surgery , Animal Welfare , Liver/surgery
Rev. colomb. cancerol ; 26(1): 111-116, ene.-mar. 2022. graf
Article in Spanish | LILACS | ID: biblio-1407973


Resumen El Xeroderma Pigmentoso (XP) es un trastorno poco frecuente, autosómico recesivo y caracterizado por una extrema sensibilidad a la radiación ultravioleta (RUV) de la luz solar, que produce una reparación defectuosa del daño del ADN y como consecuencia, marcada predisposición al desarrollo de cáncer de piel. Clínicamente, se manifiesta por fotosensibilidad, cambios cutáneos pigmentarios y envejecimiento prematuro de la piel. Este trastorno afecta aproximadamente 1 de cada 250.000 habitantes por año en Estados Unidos. Presentamos el caso de una paciente de 12 años con un Xeroderma Pigmentoso detectado a los 6 meses, quien desarrolló una lesión ulcerada en la vertiente nasal izquierda con reporte de patología de Angiosarcoma (AS) con CD31, EGR y CD34 positivos. La paciente fue tratada con resección amplia de la lesión y quimioterapia adyuvante con Plaquitaxel y Doxorrubicina sin radioterapia.

Abstract Xeroderma Pigmentosum is a rare autosomal recessive disorder characterized by extreme sensitivity to ultraviolet radiation (UVR) from sunlight that results in a defective repair of DNA damage and, as a consequence, a marked predisposition to the development of cancer of the skin. Its clinical manifestations are photosensitivity, pigmentary skin changes, and premature aging of the skin. This disorder affects approximately 1 in 250,000 individuals per year in the United States. We present the case of a 12-year-old patient with Xeroderma Pigmentosum detected at 6 months of age, who developed an ulcerated lesion on the left nasal slope with a pathology report of angiosarcoma (AS), which we positive for ERG, CD31, and CD34. The patient was treated with wide resection of the lesion and adjuvant chemotherapy with paclitaxel and doxorubicin without radiation therapy.

Male , Child , Skin Neoplasms , Xeroderma Pigmentosum , Radiotherapy , Skin , Chemotherapy, Adjuvant , Hemangiosarcoma
Clin. biomed. res ; 42(3): 292-295, 2022.
Article in Portuguese | LILACS | ID: biblio-1416964


A Síndrome de Stewart-Treves é uma rara condição onde um angiossarcoma se desenvolve sobre área de linfedema crônico. Afeta mais frequentemente os membros superiores e representa 5% dos casos de angiossarcoma. Apesar de ser mais comum em mulheres submetidas a mastectomia radical e esvaziamento axilar por neoplasia de mama, pode ocorrer a partir de linfedema de outras etiologias. Clinicamente as lesões são caracterizadas por nódulos únicos ou múltiplos de cor avermelhada ou azulada no estágio inicial e, em estágio avançado, podem evoluir para massas hemorrágicas e até gangrena. O prognóstico é ruim e a conduta cirúrgica com excisão ampla ou amputação oferecem uma maior chance de sobrevida. Relata-se um caso de Síndrome de Stewart-Treves na perna direita, apresentando-se de forma exuberante e com acometimento de linfonodos pélvicos e abdominais.

Stewart-Treves syndrome is a rare condition characterized by the development of an angiosarcoma at sites of chronic lymphedema. It most often affects the upper limbs and accounts for 5% of cases of angiosarcoma. Although the syndrome is more common in women undergoing radical mastectomy and axillary dissection for breast cancer, it may result from lymphedema of other etiologies. Clinically, the lesions are characterized by single or multiple bluish-red nodules in the early stage and may progress to hemorrhagic masses and even gangrene in advanced stages. The prognosis is poor, and surgical management with wide excision or amputation offers a greater chance of survival. We report a case of profuse Stewart-Treves syndrome in the right leg, with involvement of pelvic and abdominal lymph nodes.

Humans , Female , Middle Aged , Lower Extremity/injuries , Hemangiosarcoma/pathology , Lymphangiosarcoma/pathology , Lymphedema/complications , Amputation, Surgical
Rev. baiana saúde pública ; 45(3,supl.n.esp): 130-140, 28 dec. 2021.
Article in Portuguese | LILACS | ID: biblio-1352343


O angiossarcoma primário hepático é o tumor mesenquimal mais comum do fígado, representando cerca de 2% das neoplasias malignas primárias do órgão. Esse raro tumor tem sintomas inespecíficos, evolução agressiva e diagnóstico usualmente tardio, com prognóstico reservado mesmo quando tratado. Este trabalho consiste em um relato de caso de um paciente do sexo masculino, de 44 anos, que foi encaminhado à emergência do Hospital Geral Roberto Santos para investigação de quadro de anemia grave sintomática, síndrome consumptiva e hepatoesplenomegalia. Durante investigação laboratorial, evidenciou-se anemia com provável componente microangiopático associado à anemia da doença crônica. As sorologias para doenças virais e baciloscopia do escarro foram negativas. Foram detectados em exames de imagem dois nódulos hepáticos de grandes dimensões, adenomegalias retroperitonais, esplenomegalia de grande monta, volumoso derrame pleural à direita, além de alterações do esqueleto axial e apendicular. Evoluiu com síndrome da lise tumoral após tratamento clínico com corticoterapia por suspeita de linfoma, com óbito. A biópsia guiada por uma tomografia realizada previamente teve como conclusão perfil imuno-histoquímico compatível com angiossarcoma hepático. O angiossarcoma é um raro tumor, de difícil diagnóstico e tratamento, com evolução agressiva e achados clínico-laboratoriais pouco elucidativos, devendo a hipótese desta doença ser considerada no diagnóstico diferencial das neoplasias hepáticas. As opções terapêuticas são limitadas. Relatos de casos como este são de suma importância para o aumento do grau de suspeição clínica e um diagnóstico mais precoce dessa entidade de costumeira evolução catastrófica.

Primary hepatic angiosarcoma is the most common mesenchymal tumor of the liver, representing about 2% of primary hepatic malignancies. This rare tumor has nonspecific symptoms, delayed diagnosis, and aggressive evolution, with a poor prognosis even when treated. This study reports the case of a 44-year-old male patient referred to the emergency department of the Hospital Geral Roberto Santos with symptomatic severe anemia, consumptive syndrome, and hepatosplenomegaly. Laboratory investigation indicated anemia with a probable microangiopathic component and chronic disease anemia. Serology tests for viral diseases returned negative results, as well as sputum smear microscopy for tuberculosis. Imaging exams revealed two large hepatic nodules, retroperitoneal adenomegaly, large splenomegaly, large pleural effusion in the right lung, and bone involvement. After clinical treatment with corticosteroids for suspected lymphoma, the patient evolved with tumor lysis syndrome and died. Tomography-guided liver biopsy was previously performed, indicating an immunohistochemical profile compatible with hepatic angiosarcoma ­ a rare tumor of difficult diagnosis and treatments due to its aggressive evolution and poor clinical and laboratory findings. Considering the nonspecificity of imaging exams, this disease should be considered in the differential diagnosis of liver neoplasms investigation. Case reports such as the one described in this study are important for increasing the degree of clinical suspicion and earlier diagnosis of this malignancy.

El angiosarcoma hepático primario es el tumor mesenquimatoso del hígado más común y representa el 2% de las neoplasias malignas primarias del hígado. Este raro tumor presenta una sintomatología inespecífica, diagnóstico tardío y evolución agresiva, con mal pronóstico incluso en tratamiento. Este es un reporte de caso de un hombre de 44 años de edad, que fue remitido al servicio de urgencias del Hospital Geral Roberto Santos para investigar anemia severa sintomática, síndrome de consunción y hepatoesplenomegalia. Durante la investigación de laboratorio, se evidenció anemia con un probable componente microangiopático asociado a anemia por enfermedad crónica. La serología para enfermedades virales resultó negativa, así como la microscopía de frotis de esputo para tuberculosis. Las imágenes revelaron dos grandes nódulos hepáticos, adenomegalia retroperitoneal, gran esplenomegalia, gran derrame pleural en el pulmón derecho, así como afectación del esqueleto axial y apendicular. El paciente evolucionó con síndrome de lisis tumoral tras el tratamiento clínico con corticoides por sospecha de linfoma, y no se resistió. Previamente se realizó biopsia hepática guiada por tomografía con perfil inmunohistoquímico compatible con angiosarcoma hepático. El angiosarcoma es un tumor raro, de difícil diagnóstico y tratamiento por su evolución agresiva y deficientes hallazgos clínicos y de laboratorio. Los exámenes por imágenes son inespecíficos y la posibilidad de esta enfermedad debe considerarse en el diagnóstico diferencial de la investigación de neoplasias hepáticas. Las opciones terapéuticas son limitadas. Reportes de casos como este son importantes para incrementar el grado de sospecha clínica y el diagnóstico precoz de este tipo de evolución catastrófica habitual.

Humans , Male , Tumor Lysis Syndrome , Research Report , Anemia , Hemangiosarcoma , Liver , Liver Neoplasms
Dermatol. argent ; 27(3): 115-118, jul.- sep. 2021. il, graf
Article in Spanish | LILACS, BINACIS | ID: biblio-1372828


La proliferación vasculara típica mamaria inducida por radioterapia es una proliferación angiomatoide que aparece sobre la piel previamente irradiada por el tratamiento conservador de un carcinomademama. Se presenta el caso de una paciente de 58años que consultó por la aparición de múltiples pápulas purpúricas milimétricas en la mama derecha. Había recibido radioterapia y cuadrantectomía por un carcinoma intraductal 5años antes y estaba medicada con tamoxifeno. El análisis histópatológico e inmunohistoquímico informó: "Proliferación vascular atípica inducida por radiación, variedad atípica inducida por radiación, variedad linfática". Se adoptó una conducta expectante, con seguimiento estrecho.

Atypical vascular proliferation of the breast induced by radiation is an angiomatoid proliferation that appears on previously irradiated skin by the conservative treatament of a breast carcinoma. We present a 58-year-old female patient who consulted for multiple millimeter purpuric papules in the right breast. She received radiotherapy and quadrantectomy for an intraductal carcinoma 5 years before. She is currently on tomoxifen. The histopathology and immunohistochemistry reported atypical vascular proliferation induced by radiation, lymphatic variety. Watchuful waiting is adopted with close monitoring.

Humans , Female , Middle Aged , Breast Neoplasms , Hemangiosarcoma/diagnosis , Radiation Injuries , Tamoxifen , Neoplasm Metastasis/diagnosis
Rev. cir. (Impr.) ; 73(1): 103-106, feb. 2021. ilus
Article in Spanish | LILACS | ID: biblio-1388776


Resumen Objetivo: Comunicar el caso de una paciente de 77 años tratada de un angiosarcoma de cuero cabelludo, que evoluciona con neumotórax bilateral, cuyo estudio histopatológico informa enfermedad metastásica. Materiales y Método: Revisión de ficha clínica electrónica, registro informático de imagenología e informes de biopsias. Seguimiento ambulatorio en policlínico de cirugía. Resultados: Se realiza manejo por videotoracoscopía bilateral, con bulectomía más pleurodesis. Discusión: El neumotórax secundario por enfermedad metastásica es poco frecuente, aproximadamente 1% a 2% de los casos. De los tumores que más frecuentemente metastizan al pulmón se encuentran los sarcomas. Conclusión: El neumotórax metastásico es poco frecuente y requiere un alto índice de sospecha. Su manejo va a depender del pronóstico y de las condiciones generales del paciente.

Aim: To report the case of a 77-year-old patient treated for angiosarcoma of the scalp, who evolves with bilateral pneumothorax, whose histopathological study reports metastatic disease. Materials and Method: Review of electronic clinical record, computerized imaging record and biopsy reports. Outpatient follow-up at the polyclinic of surgery. Results: Management was performed by bilateral videothoracoscopy, with bulectomy plus pleurodesis. Discussion: Secondary pneumothorax due to metastatic disease is rare, approximately 1 to 2% of cases. Sarcomas are among the tumors that most frequently metastasize to the lung. Conclusion: Metastatic pneumothorax is infrequent and requires a high index of suspicion. Its management will depend on the prognosis and the general conditions of the patient.

Humans , Female , Aged , Pneumothorax/diagnostic imaging , Scalp/pathology , Neoplasm Metastasis/therapy , Pneumothorax/therapy , Hemangiosarcoma/complications , Lung Neoplasms/diagnostic imaging
Journal of the Philippine Dermatological Society ; : 49-52, 2021.
Article in English | WPRIM | ID: wpr-978069


Introduction@#Angiosarcoma is a rare head and neck sarcoma of vascular endothelial cell origin. We report a case of angiosar- coma in an elderly male, and the multidisciplinary approach employed in his treatment.@*Case report@#A 79-year-old male presented with a 4-month history of a rapidly enlarging black, soft, immovable tumor surround- ed by bruise-like patches over the right temporoparietal scalp. There was associated pruritus and bleeding when scratched. Dermoscopy showed bluish black crusts over the tumor, and surrounding violaceous patches. Wedge biopsy revealed a dermis with irregular vascular spaces infiltrating dermis, lined by atypical endothelial cells. Immunohistochemistry of the atypical in- filtrative cells was positive for CD31. These findings were consistent with angiosarcoma. The patient underwent wide excision with a rotational flap and split thickness skin graft. Postoperatively, the patient was referred to Oncology for adjuvant radiation therapy.@*Conclusion@#Even with treatment, the prognosis of angiosarcoma remains poor due to its aggressive nature, with a 5-year sur- vival rate ranging from 10-54%. However, early detection of the disease may increase patient survival rates. This rare case shows the importance of maintaining a high level of suspicion for lesions that have an atypical presentation to prevent delays in man- agement and improve patient outcomes.

Hemangiosarcoma , Scalp , Neoplasms
Chinese Journal of Cardiology ; (12): 374-379, 2021.
Article in Chinese | WPRIM | ID: wpr-941289


Objective: To investigate the clinical, cardiac imaging characteristics and prognosis of patients with primary cardiac angiosarcoma. Methods: The clinical data of 14 patients hospitalized with primary cardiac angiosarcoma from January 2001 to December 2017 in Peking Union Medical College Hospital were collected and analyzed. Metastatic cardiac angiosarcoma was not included in this study. Patients were followed up post discharge per telephone call or clinical visit. Results: Of the 14 patients, 8 were males and 6 were females, average age was 48 years. The main clinical symptoms were shortness of breath (8/14), hemoptysis (6/14), fever (5/14), chest pain (4/14) and cough (3/14). Imaging examinations showed that the tumors of 8 patients were located in the right heart and 6 in the pericardial cavity. Tumors in the right heart often infiltrate the atrial wall and cause pericardial effusion (7/8). Tumors in the pericardium were characterized by recurrent bloody pericardial effusion (6/6), prone to progressive constrictive pericarditis (3/6), pericardial fluid cytology was often negative (6/6). MRI showed heterogeneous high signal intensity (cauliflower aspect) on T2-weighted image and heterogeneous enhancement with a"sunray" aspect at the perfusion study. At the time of diagnosis, 8 patients developed lung or adrenal metastasis (8/14). The median survival was only 305 days. Conclusions: Primary cardiac angiosarcoma is a rare disease with non-specific clinical manifestation and poor prognosis. Imaging examinations may help diagnosis. The high invasiveness and the easy-to-metastasis feature of the tumor contribute to the poor prognosis of cardiac angiosarcoma.

Female , Humans , Male , Middle Aged , Aftercare , Heart Neoplasms/diagnostic imaging , Hemangiosarcoma/diagnostic imaging , Patient Discharge , Pericardial Effusion
Autops. Case Rep ; 11: e2020217, 2021. graf
Article in English | LILACS | ID: biblio-1142400


Angiosarcoma is a rare neoplasm, constituting only 2% of all the soft tissue tumors and most frequently involves the skin of the head and neck region in elderly males. They are extremely aggressive tumors with high rates of metastasis and poor outcomes. We report a unique case of angiosarcoma involving an unusual site - upper alveolus and maxilla in a young patient highlighting the diagnostic challenges in such a scenario. A 29 years old female presented with a non-healing wound of the oral cavity, which had progressed to the current maximum size of 6.4 cm within one month. Magnetic resonance imaging (MRI) scan revealed the involvement of maxilla up to the floor of the orbit and adjacent soft tissue. However, no distant metastasis was detected on Positron Emission Tomography (PET) scan. Biopsy of the lesion showed an irregular, highly pleomorphic, and mitotically active epithelioid soft tissue tumor conclusively diagnosed as angiosarcoma.

Humans , Female , Adult , Head and Neck Neoplasms/complications , Hemangiosarcoma , Oral Ulcer , Rare Diseases
Arq. Ciênc. Vet. Zool. UNIPAR (Online) ; 23(2, cont.): e2308, jul-dez. 2020.
Article in Portuguese | LILACS, VETINDEX | ID: biblio-1141376


O uso de técnicas de cirurgia reconstrutiva é de grande importância quando há perda de uma ampla quantidade de tecido cutâneo. O objetivo deste trabalho é relatar a utilização da técnica de retalho de avanço pediculado em decorrência de extensa ausência de tecido cutâneo resultante da exérese de neoplasia na região peniana de um cão. O presente estudo relata o caso de um cão da raça American Pitbull Terrier, macho castrado, com sete anos de idade, com histórico de neoplasia e aumento de nódulo na região peniana. Após avaliação clínica do animal, achou-se por bem o tratamento por meio da remoção cirúrgica do neoplasma. Durante o procedimento cirúrgico constatou-se que seria necessário o emprego de um retalho de avanço pediculado para fechamento da ferida ocasionada pela remoção da neoplasia. Para diminuir a tensão e os riscos de complicações no retalho, foi realizada uma "walking suture" no subcutâneo, técnicas essas que garantiram uma cicatrização com aspecto estético e funcional satisfatório com o mínimo de tensão.(AU)

The use of reconstructive surgery techniques is of great importance when there is loss of a large amount of cutaneous tissue. The objective of this study is to report the use of pedicle flap technique as a result of extensive absence of cutaneous tissue resulting from the excision of neoplasia in the penile dog region. The present study reports the case of a seven year old male Pitbull Terrier, with a history of neoplasia and nodule enlargement in the penile region. After the clinical evaluation of the animal, the treatment by the surgical removal of the neoplasm was considered good. During the surgical procedure, it was found that a pedicle flap should be used to close the wound caused by removal of the neoplasia. To reduce tension and risk of complications in the flap, a walking suture was performed on the subcutaneous tissue, which ensure healing with aesthetic and functional appearance with minimal tension.(AU)

El uso de técnicas de cirugía reconstructiva es de gran importancia cuando se pierde una gran cantidad de tejido de la piel. El objetivo de este estudio es informar el uso de la técnica de colgajo de avance pediculado debido a la extensa ausencia de tejido de la piel como resultado de la escisión de la neoplasia en la región del pene del perro. El presente estudio informa el caso de un Pitbull Terrier americano de siete años, un hombre castrado, con antecedentes de neoplasia y un nódulo agrandado en la región del pene. Después de la evaluación clínica del animal, se consideró el tratamiento mediante la extirpación quirúrgica de la neoplasia. Durante el procedimiento quirúrgico, se descubrió que el uso de un colgajo de avance pediculado sería necesario para cerrar la herida causada por la extirpación de la neoplasia. Para reducir la tensión y el riesgo de complicaciones en el colgajo, se realizó una sutura ambulante en el tejido subcutáneo, técnicas que aseguraron la curación con un aspecto estético y funcional satisfactorio con una tensión mínima.(AU)

Animals , Dogs , Plastic Surgery Procedures , Dogs/injuries , Hemangiosarcoma , Neoplasms
Arq. bras. neurocir ; 39(3): 222-227, 15/09/2020.
Article in English | LILACS | ID: biblio-1362426


Introduction Angiosarcoma (AG) is a malignant mesenchymal neoplasm that predominantly affects the soft tissues and, to variable degrees, expresses themorphological and functional characteristics of the endothelium. The incidence of sarcomas of the central nervous system(CNS) is low (0.5% to 2.7%), and AGs involving the brain are even rarer. Case Description A 45-year-old male patient presented with complaints of headache, nausea, and vomiting. An examination showed bilateral papilledema and a right lung pleurotomy. The patient's previous history included drug addiction, pulmonary tuberculosis, lung abscess, pleural empyema, and pulmonary artery embolization for severe hemoptysis. Computed tomography/magnetic resonance imaging scans revealed a large intra-axial lesion extending into the right parietal and temporal lobes, with hemorrhagic zones. The patient underwent surgical resection of the lesion. Microscopy showed a poorly-differentiated, high-grade malignant tumor composed of plump/epithelioid cells forming small vascular spaces and solid nests, compatible with AG.In the postoperative period, the patient developed recurrent hemoptysis. A biopsy of the tissues adjacent to the pleurotomy determined the diagnosis of pulmonary AG. At 30 days after the resection, the patient died from hemoptysis, hemothorax, lung atelectasis, and intracranial hypertension related to the recurrence of the brain tumor. Conclusion Angiosarcoma is a rare neoplasia related to short survival due to the high proliferative index, which must be considered in patients presenting hemorrhagic tumors. No specific genetic abnormalities have been described for this neoplasia.

Humans , Male , Middle Aged , Tuberculosis, Pulmonary/etiology , Anemia , Hemangiosarcoma/surgery , Hemangiosarcoma/complications , Prognosis , Soft Tissue Neoplasms/diagnosis , Central Nervous System Neoplasms/diagnosis , Hemangiosarcoma/physiopathology , Hemangiosarcoma/diagnostic imaging , Neoplasm Metastasis